Localized cystic disease of the kidney
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| Localized cystic disease of the kidney | |
|---|---|
| Diagnosis in short | |
|
| |
| LM |
cysts with cuboidal or flat lining epithelium - contiguous with renal collecting ducts; renal parenchyma (renal tubules or glomeruli) with in septations; +/-foamy histiocytes; +/-refractile material within the cysts |
| LM DDx | cystic renal tumours (cystic clear cell renal cell carcinoma, cystic nephroma, multilocular cystic renal neoplasm of low malignant potential), hereditary cystic renal disease (autosomal dominant polycystic kidney disease) |
| Site | kidney |
|
| |
| Signs | +/-hematuria (uncommon), often incidentaloma |
| Symptoms | abdominal pain - due to mass effect |
| Prevalence | very rare |
| Radiology | localized cystic disease, Bosniak classification 2 or 3 |
| Prognosis | benign |
| Clin. DDx | cystic renal cell carcinoma |
| Treatment | excision for diagnosis |
Localized cystic disease of the kidney is a rare benign entity that should be separated from hereditary cystic kidney diseases and cystic renal tumours.[1]
It is also known as segmental cystic disease of the kidney, unilateral cystic disease of the kidney[1] and benign multilocular cyst of the kidney.[2]
General
- Rare and benign.
- No family history of polycystic kidney disease.
Presentation:
- Usually an incidental finding (7 of 9 cases).
- Hematuria - uncommon (2 of 9 cases).
Radiology:
- Bosniak classification 2 or 3.
- No cysts in other organs - liver, pancreas.
Gross
Features:[1]
- Focal cystic lesion(s) without a capsule involving the renal papillae +/-renal cortex.
- Contain clear or light-yellow serous fluid.
Note:
- Focal - only part of the kidney should be involved.
Microscopic
Features:[1]
- Cysts with cuboidal or flat lining epithelium.
- Contiguous with renal collecting ducts.
- Renal parenchyma (renal tubules or glomeruli) with in (cyst) septations.
- +/-Foamy histiocytes.
- +/-Refractile material within the cysts.
Notes - negatives:
- No significant inflammation.
- No capsule.
- No papillary hyperplasia.
DDx:
- Cystic renal tumours.
- Hereditary cystic renal disease, e.g. autosomal dominant polycystic kidney disease.
See also
References
- ↑ 1.0 1.1 1.2 1.3 Ding, Y.; Chen, L.; Deng, FM.; Melamed, J.; Fan, R.; Bonsib, S.; Zhou, M. (Apr 2013). "Localized cystic disease of the kidney: distinction from cystic neoplasms and hereditary polycystic diseases.". Am J Surg Pathol 37 (4): 506-13. doi:10.1097/PAS.0b013e318271eff9. PMID 23211292.
- ↑ 2.0 2.1 Bhat, S.; Thomas, A. (Oct 2007). "Benign multilocular cyst or multilocular cystic nephroma in adults: three case reports.". Indian J Surg 69 (5): 209-11. doi:10.1007/s12262-007-0025-1. PMID 23132986.